Presentation
The objective is to evaluate the present status and future role of free vascular fibular transfer in treating congenital pseudoarthrosis of the tibia. [ncbi.nlm.nih.gov]
Reports described clinical presentation to range from simple anterolateral bowing of tibia to frank pseudarthrosis 3 .In this series 25 out of 37(60%) presented with established pseudarthrosis, while 10 out of 37(27%) presented with bowing. [sudjms.net]
Presenting problem: The table summarises patient characteristics, presenting problem, safeguarding assessment and action taken by social services. Café-au-lait lesions were present in all 3 patients. [bone-abstracts.org]
True bone dysplasia with non union or potential non union through a hamartomatoUsually area in the tibia. not present at birth really a fibrous non-union of a pathological fracture The defect in the bone is filled with mature, fibrous connective tissue [orthofracs.com]
Entire Body System
- Noncompliance
[…] significantly higher with a younger age at surgery (less than 4 years), low cross-sectional area of the healed segment, recurrence of tumor, when associated with persistent fibular pseudarthrosis, residual ankle valgus, removal of an intramedullary rod, and noncompliance [ijoonline.com]
Neurologic
- Dysesthesia
Recent studies show that donor-site morbidity after harvest of a free osteocutaneous fibula segment is low. 38,39 The most frequent complaints are pain, dysesthesia, and a feeling of ankle instability. [jpma.org.pk]
- Forgetful
[…] compression healed 38 Treatment Type III Closed distraction Gradual deformity correction Lengthening IM Rod (late) 39 distraction of CPT follow-up 40 RESULTS Periosteal Bone Grafting with IM rod and Ilizarov 1997-2008 20 patients treated Union 20/20 41 Dont Forget [powershow.com]
Treatment
The authors followed up 9 cases of congenital pseudoarthrosis associated with neurofibromatosis-1 (or Von Recklinghausen's disease) at least 2 years after the end of treatment using Ilizarov's method. [moh-it.pure.elsevier.com]
Conventional treatments used included excision of the pseudoarthrosis, correction of the deformity, intramedullary fixation, bone grafting and soft tissue transplantation for lengthening and coverage. [ncbi.nlm.nih.gov]
and union after one additional treatment in 1 child. [42] Mean age at the time of treatment was 8 years. [ijoonline.com]
Prognosis
Prognosis continues to be reserved especially in clepsydra forms and those localised to the lower third of the tibia. [moh-it.pure.elsevier.com]
The prognosis of CPT is very much related to the radiologic classification. Crawford type 2 and Crawford type 4 pseudoarthrosis have a worse prognosis, with a lower percentage of fusion at the site of pseudoarthrosis. [ncbi.nlm.nih.gov]
Type 2 have a relative poor prognosis. Type 3 cases are usually of late onset type which develops as a stress fracture of congenital dysplastic tibia or in a healed tibia after previous bone grafting & has the best prognosis. [de.slideshare.net]
Prognosis worse with Neurofibromatosis 1/2 of cases 3 Fracture develops at site of bone cyst Usually near junction of mid/ distal 1/3 Anterior bowing precedes or follow fracture 4 Sclerotic bony segment initially present at junction of mid/ distal 1/ [orthofracs.com]
The prognosis is good if the lesion is located only on the fibula, an extension to the tibia has a prognosis similar to type II, Type VI Associated with an intraosseous fibroma or a schwannoma. [boneandspine.com]
Etiology
Abstract The purpose of study was to refine frequency of etiological factors and characteristics of congenital pseudarthrosis of tibia (CPT). Materials and Methods. [journals.eco-vector.com]
Congenital pseudoarthrosis of the tibia (CPT) is an uncommon disease whose etiology and pathogenesis is unknown. [ncbi.nlm.nih.gov]
Pathology The etiology is unclear, however, around 50% of cases are associated with neurofibromatosis type 1 (NF1). Overall, 10% of patients with NF1 are diagnosed with tibial pseudoarthrosis. [radiopaedia.org]
Etiology Each type of tibial bowing tends to have its own etiology. [emedicine.medscape.com]
The etiological nature of this lesion is unclear, however there is a strong association between CPT and neurofibromatosis type I (NF-I). [jlponline.org]
Epidemiology
Congenital pseudarthrosis of the tibia: history, etiology, classification, and epidemiologic data. J Pediatr Orthop B 2000; 9:11-15 [Google Scholar] 5. Grill F, Bollini G, Dungl P, et al. [ajronline.org]
Horn J, Steen H, Terjesen T (2013) Epidemiology and treatment outcome of congenital pseudarthrosis of the tibia. J Child Orthop 7(2):157–166 PubMedCentral CrossRef PubMed 20. [springermedizin.de]
Epidemiological data are limited, and treatment of the condition is challenging. The purpose of our study was to gain epidemiological data on the incidence of CPT in Norway and to evaluate the treatment outcome of the disease. [readbyqxmd.com]
Bowing usually at junction of Mid / Distal 1/3 Epidemiology Rare 1:200 000 M = F 50% have Neurofibromatosis type 1 1-3% of Neurofibromatosis have CPT Aetiology Cause unknown Theories include: Intrauterine fracture Localised vascular abnormality Constriction [orthofracs.com]
Introduction Define/describe the condition Anatomy Describe the pertinent anatomy Pathogenesis Describe the biomechanics/biologic basis of the disorder or the mechanism of injury Natural History Describe the natural history, epidemiology and prognosis [orthopaedicsone.com]
Pathophysiology
Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am 2005;87:1848-64. Wang K, Allen L, Fung E, et al. Bone scintigraphy in common tumors with osteolytic components. Clin Nucl Med 2005;30:655-71. Tuncel M, Kiratli PO, Gedikoglu G. [journals.sfu.ca]
A relationship with type 1 neurofibromatosis or von Recklinghausen’s disease is known but exact pathophysiology has still not been clearly defined. [boneandspine.com]
Appropriate interpretation will improve understanding of the natural history and pathophysiology of neurofibromatosis type 1. [nature.com]
The pathophysiology of the resultant limb-length inequality may be related to the bowing. [emedicine.medscape.com]
Prevention
At the end of the correction, a telescopic Fassier-Duval intramedullary nail was used prophylactically to prevent re-fracture. After 2 years of follow-up, X-ray images show bone-healing without any bowing. [ncbi.nlm.nih.gov]
Moreover it does not always prevent ankle valgus [Figure 4]. To prevent all these, the rod must be kept till skeletal maturity. [ijoonline.com]
Created to prevent or stabilize fractures, or correct deformity of long bones whilst growth occurs. [pegamedical.com]
poor protection against re-fracture, failure to correct limb length discrepancy, and deformities of leg and ankle simultaneously at time of primary surgery. 8 Vascularized fibular graft/ Nail The intramedullary rod offers good tibial alignment and prevents [slideplayer.com]